Chronic lymphatic-system failure (lymphedema) occurs in 3-5 million Americans across a wide spectrum of diseases.
- Primary (congenital)
- Secondary (acquired)
Primary
Over 40 rare diseases are associated with primary lymphedema including:
- Aagenaes Syndrome
- Adams-Oliver Syndrome
- C.H.A.R.G.E. Syndrome
- C.L.O.V.E.S. Syndrome
- Carbohydrate Deficient Glycoprotein (types 1a, 1b, 1h)
- ardio-facial-cutaneous Syndrome
- Choanalatresia-lymphedema Syndrome
- Congenital Lymphedema (non-Milroy’s)
- Ectodermal Dysplasia Anhidrotic
- Immunodeficiency Osteopetrosis
- Lymphedema Syndrome
- Fabray’s Disease
- Gorham’s Disease
- Hennekam Syndrome
- Hypotrichosis Lymphedema Telangiectasia
- Klippel Trenaunay Syndrome
- Klippel-Trenaunay-Weber Syndrome
- Lipedema
- Lymphedema Distichiasis Syndrome
- Lymphedema Myelodysplasia (Emberger Syndrome)
- Lymphedema Praecox
- Lymphedema Tarda
- Lymphedema-Distichiasis
- Macrocephaly-Capillary Malformation
- Maffucci Syndrome
- Meige Syndrome
- Microcephaly-Chorioretinopathy-Lymphedema-Mental Retardation Syndrome
- Milroy’s Disease
- Mucke Syndrome
- Neurofibromatosis
- Nonne-Milroy Disease
- Noonan’s Syndrome
- Oculo-Dento-Digital Syndrome
- Prader Willi Syndrome
- Progressive Encephalopathy-Hypsar-rhythmia-Optic Atrophy Syndrome
- Protein Losing Enteropathy (associated with numerous forms of congenital heart disease)
- Proteus Syndrome
- Spina bifida
- Thrombocytopenia with Absent Radius Syndrome
- Trisomy 13, 18, 21
- Turner’s Syndrome
- Velocardiofacial Syndrome
- W.I.L.D. Syndrome
Secondary
Secondary cases can be broken into two categories:
- Non-Cancer
- Cancer
22% of all cases are non-cancer related. Any significant damage to the lymphatic organ system can result in lymphedema. Cases include:
- Burns
- Ilio-femoral bypass
- Infection
- Paralysis
- Radiation
- Rheumatoid arthritis
- Surgery
- Trauma
68% of all cases are cancer related. The overall cancer-related incidence rate is 15.5%. Specific rates include:
- Breast 40%
- Sarcoma 30%
- Gynecological 20%
- Melanoma 16%
- Genital-urinary 10%
- Head and neck 4%
SOURCE
Lymphedema: Not One Disease. LymphedemaTreatmentAct.org, lymphedematreatmentact.org/wp-content/uploads/2018/02/Lymphedema-Treatment-Act-HR930_S497.pdf.
